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Males typically have one x chromosome and one y chromosome in each diploid cell of their bodies Xx male syndrome, also known as de la chapelle syndrome, is a rare genetic condition where an individual has a typical male phenotype (external male characteristics) but possesses two x chromosomes instead of the usual xy configuration. Females typically have two x chromosomes.

There are two kinds of sex chromosomes Testis biopsy usually reveals an absence of spermatogenesis. Sex chromosomes decide whether you’re biologically male (xy) or female (xx), but they don’t define your gender.

Xx male syndrome is a structural and numerical chromosomal condition that presents as a male with azoospermia

Typically, there is normal male external and internal genitalia

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